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     1  SARCOIDOSIS Rheumatology Module Anna Mae Smith, MPAS, PA-C
     2  Definition Multisystem granulomatous disorder What’s granulomatous??? Mass or nodule composed of chronically inflamed tissue. Non-infectious multisystem disease of unknown cause, commonly affecting young and middle-age adults.
     3  Presentation Bilateral symmetrical hilar lymphadenopathy Pulmonary infiltrates Skin lesions Eye lesions(uveitis) Other organs may be involved, including liver, spleen, lymph nodes, heart, and central nervous system.
     4  Immunopathology Sarcoid granulomas consist of focal accumulations of epitheloid cells, macrophages, lymphocytes, mainly T cells! T lymphocytes…slightly B cells in the circulatory system.
     5  Epidemiology Increased prevalence found in Scandinavians, Japanese, Irish females, and African American women 30-80 per 100,000 Predominant age: 20-60 years Women > men Course worse in African Americans than whites
     6  Differential Diagnosis Infectious granulomatous disease such as tuberculosis and fungal infections Foreign body reactions Lymphoma Other malignancies associated with lymphadenopathy Berylliosis
     7  Presentation Present with pulmonary symptoms or found on x-ray! Fatigue Weight loss
     8  Presentation Cough Shortness of breath Skin (new lesions) Pain or irritation of eyes General fatigue, malaise Fever Night sweats Bell's palsy
     10  Bilateral hilar lymph adenopathy Characteristic feature of sarcoid Often symptomless but may have associated dull, ache in chest, malaise & fever
     11  Pulmonary Infiltration More progressive & leads to dyspnea…cor pulmonale…death Honeycomb appearance on chest x-ray! PFT’s restrictive pattern
     13  Skin Erythema nodosum Bilateral symmetrical hilar lymphadenopathy + erythema nodosum = sarcoidosis!!!
     15  Bone & Joint Arthralgias Bone cysts (especially in the digits) Swelling
     17  Acute Sarcoid Arthritis Migratory, Additive, Symmetric Polyarthritis (usu. Ankles & knees) Monoarthritis very rare Minimal effusion Minimal Inflammation Synovial Bx- Mild inflammation
     18  Cardiac Ventricular dysrhythmias Conduction defects Cardiomyopathy with CHF Hypercalcemia
     19  labs X-ray CBC - normochromic, normocytic anemia, Lymphopenia, leukopenia Chem20 - calcium & hypergammaglobulinemia Bronchial Biopsy ACE PFT
     20  CXR staging Routine chest roentgenograms are staged using Scadding's classification: Stage 0 = normal Stage 1 = hilar adenopathy alone Stage 2 = hilar adenopathy plus parenchymal infiltrates Stage 3 = parenchymal infiltrates alone Stage 4 = pulmonary fibrosis
     21  Special Tests Abnormal liver function, especially increased alkaline phosphatase is frequently encountered Hypercalciuria occurs in up to 10% of patients, with hypercalcemia less frequent Kveim-Siltzbach skin test can be performed. This test is fairly sensitive (approximately 80%) and highly specific (> 95%)
     22  Be careful... Prednisone will lower serum-angiotensin converting enzyme Hyperthyroidism and diabetes will increase serum angiotensin converting enzyme level
     23  Treatment Hilar lymphadenopathy alone with no evidence of lung parenchymal involvement…NO treatment! Steroids!! 30mg qd x 6 wks than 15mg qd x 6-12 months Topical too!
     24  Alternative Drugs Methotrexate 10 mg/wk hydroxychloroquine (Plaquenil) 100-200 mg per day azathioprine 50-100 mg per day chlorambucil 0.1-0.2 mg/kg.
     25  Prognosis If bilateral hilar lymphadenopathy…80% remission rate 80% of patients will have spontaneous resolution within two years 10% will have significant fibrosis but no further worsening of disease after 2 yrs 10% (higher in some populations, including African Americans) will have chronic dz.